AB0579 EVALUATION OF PATIENTS WITH PEDIATRIC BEHÇET’S DISEASE: A TERTIARY CENTER EXPERIENCE
Authors: Z. Karali, Ş. Çekiç, I. Çakir, S. S. Kilic
Journal: Annals of the Rheumatic Diseases (2022)

Background Behçet’s disease (BD) is an systemic inflammatory vasculitis characterized by recurrent oral aphthae and genital ulcers. In the course of the disease, skin, eye, musculoskeletal, nervous system and gastrointestinal system involvements can be detected. Objectives To evaluate the clinical, laboratory and radiological findings of pediatric cases diagnosed with BD. Methods Fifty patients (0-20 years old) who were followed up with the diagnosis of BD at the Pediatric Rheumatology outpatient clinic of Uludag University Faculty of Medicine between January 2011 and July 2021 were included in our study. The patients were diagnosed according to the diagnostic criteria of the International BD Study Group. Results Twenty-four (48%) of the patients were male and 26 (52%) were female. The mean age at the diagnosis of patients was 9 ±4.55 years (10.75±4.55 years in boys, 12.35±3.65 years in girls). Twenty patients (36.3%) had a family history of Behçet’s Disease. Oral aphthae were present in 96% (n=48) patients, while genital ulcers were in 32% (n=16) (Table 1). Of the nine patients with uveitis, 6 had panuveitis, 2 had anterior uveitis and 1 had posterior uveitis. . There was no difference in the distribution of symptoms according to the gender of the patients. HLA-B51 allel was found in 39 (78%) and ANA was positive in 14 patients (28%). Immunological tests showed that serum immunoglobulins were low in 11 (32.3%) of 34 patients.Low IgG levels were detected in 6 patients, low IgM levels were in 3, and low IgA levels were in 2. Thrombus was presented in three cases (thrombus in the right ventricle in one case, in the intracranial transverse sigmoid sinus and left jugular vein in two cases). The most commonly used drug for aphthae was colchicine (n=45, 82%). The use of biological agents according to patient manifestations is shown in Table 2. In the follow-up, clinical findings improved in 35 patients (70%)Complete improvement was detected only with biological agents in 8 patients with uveitis. One patient was operated due to the development of complicated cataracts secondary to uveitis. Three patients were diagnosed with Covid-19, one of them was followed without treatment, while two of them were treated with favipiravir at home. Three patients with Covid-19 infection were using only colchicine treatment. Table 1. Findings at the time of diagnosis n % Uveitis 9 18 Skin findings 18 36 Gastrointestinal tract involvement 19 38 Central nervous system involvement 6 12 Vascular involvement 3 6 Arthritis 17 34 Table 2. Findings Biological Agents Uveitis (n) Arthritis (n) Venous thrombus (n) Central nervous system involvement (n) Azathioprine 2 2 3 1 Methotrexate 2 2 - - Adalimumab 3 1 - - Infliximab 3 1 - - Conclusion Behçet’s disease is rare in childhood. Although it is not common, life-threatening complications can be observed. To reduce morbidity and complications, physicians should be aware of manifestations and rare clinical pictures of the BD. References [1]Hu YC, Chiang BL, Yang YH. Clinical Manifestations and Management of Pediatric Behçet’s Disease. Clin Rev Allergy Immunol. 2021 Oct;61(2):171-180. [2]Costagliola G, Cappelli S, Consolini R. Behçet’s Disease in Children: Diagnostic and Management Challenges. Ther Clin Risk Manag. 2020 Jun 11;16:495-507. [3]Balt J, Jamyanjav B, Jav S, Dandii Z, Ganbold C, Horie Y, Lennikov A, Uehara O, Ohno S, Kitaichi N. Clinical features of Behcet’s disease in Mongolia: a multicenter study. Clin Rheumatol. 2020 Sep;39(9):2697-2706. [4]Muruganandam M, Rolle NA, Sibbitt WL Jr, Cook GB, Emil NS, Fangtham M, Reiter KJ, Bankhurst AD. Characteristics of Behcet’s Disease in the American Southwest. Semin Arthritis Rheum. 2019 Oct;49(2):296-302. Disclosure of Interests None declared